This study aims to identify neuropsychiatric manifestations in neurological Wilson disease (NWD), and their correlation with MRI changes and glutamate excitotoxicity. Forty-three consecutive patients with NWD from a tertiary care teaching hospital were evaluated prospectively who fulfilled the inclusion criteria. The neuropsychiatric evaluation was done using Neuropsychiatric Inventory (NPI) battery that assesses 12 domains including delusion, hallucination, agitation/aggression, dysphoria/depression, anxiety, euphoria, apathy, disinhibition, irritability, aberrant motor activity, appetite change, and abnormal nighttime behavior. Cranial MRI was done using a 3 T machine, and locations of signal changes were noted including the total number of MRI lesions. Serum glutamate level was measured by a fluorescence microplate reader. Abnormal NPI in various domains and total NPI scores were correlated with MRI lesions, serum and urinary copper, and glutamate level. The median age of the patients was 16 years. Forty-one (48.8%) patients had cognitive impairment and 37 (86%) had movement disorder. Neurobehavioral abnormality was detected in all—commonest being agitation (90.7%) followed by appetite change (81.4%), elation (74.4%), irritability (69.8%), anxiety (67.4%), depression (65.1%), apathy (44.2%), night time abnormal behavior (32.6%), aberrant motor behavior (20.9%), delusions (16.3%), and hallucination (18.6%). The thalamic lesion was associated with depression, globus pallidus with depression and anxiety, caudate with anxiety and agitation, brainstem with irritability, and frontal cortex with apathy. Serum glutamate level was higher in NWD. NPI sum score correlated with MRI load and glutamate level. Varying severity of neurobehavioral abnormalities are common in the patients with NWD and correlate with the location of MRI lesion and glutamate level.

本研究旨在确定神经系统威尔逊病 (NWD) 的神经精神表现，以及它们与 MRI 变化和谷氨酸兴奋性毒性的相关性。对来自一家三级护理教学医院的连续 43 名 NWD 患者进行了前瞻性评估，他们符合纳入标准。神经精神病学评估是使用神经精神病学清单 (NPI) 电池进行的，该电池评估 12 个领域，包括妄想、幻觉、激动/攻击、烦躁/抑郁、焦虑、欣快、冷漠、去抑制、易怒、异常运动活动、食欲变化和异常夜间行为. 使用 3 T 机器进行颅脑 MRI，并记录信号变化的位置，包括 MRI 病灶的总数。通过荧光酶标仪测量血清谷氨酸水平。不同领域的异常 NPI 和总 NPI 评分与 MRI 病变、血清和尿铜以及谷氨酸水平相关。患者的中位年龄为 16 岁。41 名 (48.8%) 患者有认知障碍，37 名 (86%) 有运动障碍。所有人都检测到神经行为异常——最常见的是激动（90.7%），其次是食欲改变（81.4%）、兴高采烈（74.4%）、易怒（69.8%）、焦虑（67.4%）、抑郁（65.1%）、冷漠（44.2） %)、夜间异常行为 (32.6%)、异常运动行为 (20.9%)、妄想 (16.3%) 和幻觉 (18.6%)。丘脑病变与抑郁有关，苍白球与抑郁和焦虑有关，尾状核与焦虑和激动有关，脑干与易怒有关，额叶皮质与冷漠有关。NWD的血清谷氨酸水平较高。NPI总分与MRI负荷和谷氨酸水平相关。NWD 患者的神经行为异常的严重程度不同是常见的，并且与 MRI 病变的位置和谷氨酸水平相关。